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Blood, 15 June 2008, Vol. 111, No. 12, pp. 5496-5504.
Prepublished online as a Blood First Edition Paper on April 2, 2008; DOI 10.1182/blood-2008-01-134270.


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CLINICAL TRIALS AND OBSERVATIONS

ALK anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project

Kerry J. Savage1, Nancy Lee Harris2, Julie M. Vose3, Fred Ullrich4, Elaine S. Jaffe5, Joseph M. Connors1, Lisa Rimsza6, Stefano A. Pileri7, Mukesh Chhanabhai8, Randy D. Gascoyne8, James O. Armitage3, Dennis D. Weisenburger, for the International Peripheral T-Cell Lymphoma Project9

1 Department of Medical Oncology, British Columbia Cancer Agency, Vancouver, BC; 2 Department of Pathology, Massachusetts General Hospital, Boston; Departments of3 Internal Medicine and 4 Preventive and Societal Medicine, University of Nebraska Medical Center, Omaha; 5 Department of Hematopathology, National Cancer Institute, Bethesda, MD; 6 Department of Pathology, University of Arizona, Tucson; 7 Department of Pathology, Bologna University School of Medicine, Bologna, Italy; 8 Department of Pathology, British Columbia Cancer Agency, Vancouver, BC; and 9 Department of Pathology, University of Nebraska Medical Center, Omaha

The International Peripheral T-Cell Lymphoma Project is a collaborative effort designed to gain better understanding of peripheral T-cell and natural killer (NK)/T-cell lymphomas (PTCLs). A total of 22 institutions in North America, Europe, and Asia submitted clinical and pathologic information on PTCLs diagnosed and treated at their respective centers. Of the 1314 eligible patients, 181 had anaplastic large-cell lymphoma (ALCL; 13.8%) on consensus review: One hundred fifty-nine had systemic ALCL (12.1%) and 22 had primary cutaneous ALCL (1.7%). Patients with anaplastic lymphoma kinase–positive (ALK+) ALCL had a superior outcome compared with those with ALK ALCL (5-year failure-free survival [FFS], 60% vs 36%; P = .015; 5-year overall survival [OS], 70% vs 49%; P = .016). However, contrary to prior reports, the 5-year FFS (36% vs 20%; P = .012) and OS (49% vs 32%; P = .032) were superior for ALK ALCL compared with PTCL, not otherwise specified (PTCL-NOS). Patients with primary cutaneous ALCL had a very favorable 5-year OS (90%), but with a propensity to relapse (5-year FFS, 55%). In summary, ALK ALCL should continue to be separated from both ALK+ ALCL and PTCL-NOS. Although the prognosis of ALK ALCL appears to be better than that for PTCL-NOS, it is still unsatisfactory and better therapies are needed. Primary cutaneous ALCL is associated with an indolent course.


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